title: Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature.
abstract: OBJECTIVE
  To describe the clinical presentation of the association between pulmonary fibrosis (PF) and systemic vasculitis related to antineutrophil cytoplasmic antibodies (ANCA-V).
  
  
  METHODS
  12 patients (three female, mean age 70.7 years) with ANCA-V associated with "idiopathic" PF were studied retrospectively.
  
  
  RESULTS
  ANCA-V and PF were diagnosed simultaneously in eight cases; PF occurred earlier in three cases and during ANCA-V follow-up in one. No patient had intra-alveolar haemorrhage (IAH). ANCA were myeloperoxidase (MPO)-ANCA in all cases. Seven patients had blood eosinophilia at diagnosis. Two patients died during ANCA-V induction therapy. The respiratory status of five patients worsened and three of them died from exacerbation of end-stage respiratory failure. The five remaining patients had a stable respiratory status.
  
  
  CONCLUSIONS
  The association of PF and ANCA-V does not seem to be fortuitous, even though their clinical evolutions are clearly not related. PF was the major cause of death.
authors.affiliation: Department of Internal Medicine, CHU Nantes 44093, France. baptiste.hervier@chu-nantes.fr
authors.forename: B
authors.initials: B
authors.lastname: Hervier
authors.forename: C
authors.initials: C
authors.lastname: Pagnoux
authors.forename: C
authors.initials: C
authors.lastname: Agard
authors.forename: J
authors.initials: J
authors.lastname: Haroche
authors.forename: Z
authors.initials: Z
authors.lastname: Amoura
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.forename: M A
authors.initials: MA
authors.lastname: Hamidou
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000209:etiology* / Q000276:immunology; D012189:Retrospective Studies; D014657:Vasculitis / Q000150:complications* / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic